Shen Weimin, Cui Jie, Chen Jianbin, Zou Jijun, Xiaoying Zhang
From the *Third Affiliated Hospital of Soochow University, Changzhou, China; and †Department of Plastic Surgery, Nanjing Children's Hospital, Nanjing Medical University, Nanjing, China.
J Craniofac Surg. 2014 Nov;25(6):2188-91. doi: 10.1097/SCS.0000000000001145.
Kasabach-Merritt phenomenon (KMP) is characterized by thrombocytopenia, microangiopathic hemolytic anemia, consumptive coagulopathy, and an enlarging vascular lesion. It is a rare and life-threatening disease of vascular tumor. The purpose of this study was to assess the effectiveness of absolute ethanol in the treatment of KMP.
We treated 8 pediatric patients with KMP by using absolute ethanol injections, whose cases failed previously when applied steroid therapy. We reviewed the clinical and laboratory data of these 8 cases at Nanjing Children's Hospital in China.
Eight pediatric patients (5 female and 3 male) showed tumor regression after treatments. Core needle biopsy had been performed on the 8 patients, and the results included 7 kaposiform hemangioendotheliomas and one tufted angioma. All patients had an increase in platelet count. All coagulopathies were corrected. Complications included one patient with flush on face, one patient with needle scars, and one patient with a small area of local tissue necrosis.
Absolute ethanol therapy presents a safe option in the treatment of KMP. Treatment-associated complications seemed to be reversible and acceptable by severity levels. Direct intralesional injection of absolute ethanol provides a simple and reliable alternative treatment for KMP among infants and may be used as the second-line therapy.
卡萨巴赫-梅里特现象(KMP)的特征为血小板减少、微血管病性溶血性贫血、消耗性凝血病以及血管病变不断增大。它是一种罕见且危及生命的血管肿瘤疾病。本研究的目的是评估无水乙醇治疗KMP的有效性。
我们对8例KMP患儿采用无水乙醇注射治疗,这些患儿先前应用类固醇疗法无效。我们回顾了中国南京儿童医院这8例病例的临床和实验室数据。
8例儿科患者(5例女性,3例男性)治疗后肿瘤缩小。对这8例患者进行了粗针活检,结果包括7例卡波西样血管内皮瘤和1例簇状血管瘤。所有患者的血小板计数均升高。所有凝血病均得到纠正。并发症包括1例面部潮红患者、1例有针痕患者和1例局部小面积组织坏死患者。
无水乙醇疗法是治疗KMP的一种安全选择。与治疗相关的并发症似乎是可逆的,且根据严重程度是可接受的。无水乙醇直接瘤内注射为婴儿KMP提供了一种简单可靠的替代治疗方法,可作为二线治疗。